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原发性纤毛在含牙囊肿发病机制中的作用:基于原发性纤毛在常染色体显性多囊肾病中作用的新假说

Primary cilia in the pathogenesis of dentigerous cyst: a new hypothesis based on role of primary cilia in autosomal dominant polycystic kidney disease.

作者信息

Anoop U R, Verma Kavita, Narayanan K

机构信息

K. K. Uthaman Dental Care and Implant Centre, Kidney Centre, Pondicherry Medical Mission, Pondicherry, India.

出版信息

Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011 May;111(5):608-17. doi: 10.1016/j.tripleo.2010.12.016. Epub 2011 Mar 24.

DOI:10.1016/j.tripleo.2010.12.016
PMID:21439862
Abstract

Autosomal dominant polycystic kidney disease (ADPKD), an inherited disease, leads to cyst formation in the kidneys. In this condition, the kidneys are grossly enlarged with multiple cysts that result in kidney failure in a majority of individuals. This condition is also associated with cysts in other organs. Recent research has focused on defects in signaling mediated by the primary cilia as the causative factor in ADPKD. Primary cilia are also present in odontogenic epithelium. Dentigerous cyst also is a developmental cyst whose pathogenesis is controversial. Recent studies have shown that loss of Ptch and Shh signaling pathways are involved in the cystogenesis of dentigerous cyst. The Shh signaling pathway is active in the primary cilia. A scanning electron microscopic study of a dentigerous cyst wall in an ADPKD patient showed structures similar to primary cilia. Based on the presentation of a dentigerous cyst in an autosomal dominant polycystic kidney patient and the demonstration of primary cilia like structures on the cyst wall by using a scanning electron microscope, a new hypothesis for the pathogenesis of dentigerous cyst is proposed.

摘要

常染色体显性多囊肾病(ADPKD)是一种遗传性疾病,可导致肾脏形成囊肿。在这种情况下,肾脏会明显肿大,出现多个囊肿,大多数患者会因此发展为肾衰竭。这种疾病还与其他器官的囊肿有关。最近的研究集中在原发性纤毛介导的信号缺陷是ADPKD的致病因素。原发性纤毛也存在于牙源性上皮中。含牙囊肿也是一种发育性囊肿,其发病机制存在争议。最近的研究表明,Ptch和Shh信号通路的缺失与含牙囊肿的囊肿形成有关。Shh信号通路在原发性纤毛中活跃。一项对ADPKD患者含牙囊肿壁的扫描电子显微镜研究显示了类似于原发性纤毛的结构。基于常染色体显性多囊肾病患者中含牙囊肿的表现以及通过扫描电子显微镜在囊肿壁上显示出类似原发性纤毛的结构,提出了含牙囊肿发病机制的新假说。

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引用本文的文献

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Genetic analysis and counseling of ADPKD caused by novel heterozygous mutations of in two Chinese families: Case report.两个中国家系中由新型杂合突变引起的常染色体显性多囊肾病的基因分析与遗传咨询:病例报告
Heliyon. 2024 Nov 15;10(22):e40407. doi: 10.1016/j.heliyon.2024.e40407. eCollection 2024 Nov 30.
2
Primary cilia and hypoxia-associated signaling in developmental odontogenic cysts in relation to autosomal dominant polycystic kidney disease - A novel insight.原发性纤毛与发育性牙源性囊肿中与常染色体显性多囊肾病相关的缺氧信号传导——一项新见解
Heliyon. 2023 Jun 9;9(6):e17130. doi: 10.1016/j.heliyon.2023.e17130. eCollection 2023 Jun.
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Primary Cilia: The New Face of Craniofacial Research.
原发性纤毛:颅面研究的新面貌。
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From bone abnormalities to mineral metabolism dysregulation in autosomal dominant polycystic kidney disease.从骨骼异常到常染色体显性多囊肾病中的矿物质代谢失调。
Pediatr Nephrol. 2013 Nov;28(11):2089-96. doi: 10.1007/s00467-012-2384-5. Epub 2013 Jan 24.